Transfer factor therapy in the Wiskott-Aldrich syndrome. Results of long-term follow-up in 32 patients.

Thirty-two patients with the Wiskott-Aldrich syndrome have been treated with transfer factor provided by this laboratory. Apparent clinical benefit was observed in 44 per cent of them. The mean age of the patients who showed clinical benefit was significantly greater than that of the patients who showed no benefit. Conversion of immunologic reactivity correlated with clinical benefit. Thirteen of the patients who received transfer factor are alive, and 17 have died (43 per cent survival). Clinical benefit was correlated with survival. The median survival was greater than five years in the patients who showed clinical benefit, whereas it was 18 months in those who did not show clinical benefit. We conclude that transfer factor caused conversion of immunologic parameters, apparent clinical benefit and prolonged survival in some, but not all, patients with the Wiskott-Aldrich syndrome.     

Clinical features of patients with Legionnaires disease showing initial clinical improvement but radiological deterioration: A retrospective single-center analysis

Patients with Legionnaires disease occasionally experience initial clinical improvement but radiological progression. However, data on this issue are so far limited. The aim of this study was to investigate changes in chest radiograph findings in patients with Legionnaires disease who showed initial clinical improvement and to identify risk factors and outcomes in these patients.All patients diagnosed with Legionnaires disease at a tertiary hospital in South Korea between March 2011 and May 2020 were retrospectively enrolled. Legionnaires disease was defined as abnormal chest radiographs accompanied by a positive finding on at least one of the following tests: urinary antigen test, sputum Legionella polymerase chain reaction, and sputum Legionella culture. Clinical improvement was defined as defervescence and decreased C-reactive protein level. Clinical and radiological records were reviewed on treatment days 7 and 14 and at discharge. We describe the characteristics of patients with clinical improvement but radiological deterioration on treatment for Legionnaires disease and compared them with patients with initial clinical improvement and stable or resolving chest radiograph findings.Of 140 patients with Legionnaires disease, 33 (24%) showed initial clinical deterioration, while the remaining 107 (76%) showed initial clinical improvement on day 7. The latter 107 patients were analyzed in this study; 22 (21%) showed radiological progression despite the clinical improvement. Risk factors for these patients were a high pneumonia severity index score and the use of mechanical ventilation. Mortality did not significantly differ between those with initial clinical improvement but radiological deterioration and those with both initial clinical and radiological improvement (28% vs 12%, P = .49).About one-fifth of patients with Legionnaires disease, especially those who had a high pneumonia severity index score and underwent mechanical ventilation, showed radiological deterioration despite of clinical improvement 1 week after appropriate treatment, while outcomes were not significantly worse in these patients. Therefore, our findings support that close monitoring without modification of antibiotics use is warranted in those who have clinical improvement regardless of radiologic findings.     

鸡源性肠炎沙门氏菌的鉴定

摘要：目的 本次试验主要是运用多种方法鉴定临床分离的一株沙门氏菌。方法 这次试验从细菌培养特性、生化特性方面来鉴定临床分离菌属于那类细菌;测定临床分离菌的血清型根据血清型试验结果进一步确定临床分离菌的类型;测定临床分离菌的16s rRNA 从分子生物学角度鉴定细菌;应用多位点序列分型（MLST）对该株细菌进行分型。结果 从临床分离的沙门氏菌与肠炎沙门氏菌的微生物学和生物化学特征相符合,血清学试验结果确定其血清型为O3,19(+);O9(+);Hg,p(+);Hg(+);Hm(+),16s RNA基因测序结果显示该株细菌与鸡肠炎沙门氏菌P125109同源性达100%,多位点序列分型结果显示该株细菌基因型为ST11。结论 该株细菌为肠炎沙门氏菌血清型为O3,19(+);O9(+);Hg,p(+);Hg(+);Hm(+),基因型为ST11。     

Systemic sclerosis (scleroderma). A case of recovery of cardiomyopathy after vitamin E treatment

A 60-year-old woman with systemic sclerosis, systemic hypertension, and chronic renal failure, presented with clinical manifestations of heart failure. An echocardiogram showed a mildly dilated left ventricle and global hypokinesis. A six-month treatment including reduced sodium intake, furosemide, and nifedipine did not change the clinical and instrumental findings. Casually, vitamin E (600 mg daily) was added. After 6 months, clinical manifestations of heart failure were disappeared and the echocardiogram showed a normally-sized left ventricle with normal wall motion.     

Heterogeneity of prognosis in adult IgA nephropathy, especially with mild proteinuria or mild histological features

OBJECTIVE: The present study was undertaken to clarify the clinical course and prognosis of adult patients with primary IgA nephropathy (IgAN), especially with mild proteinuria or mild histological alternations. PATIENTS AND METHODS: A population of 735 IgAN patients whom we were able to observe for more than two years was examined. RESULTS: A total of 115 patients (15.6%) was on dialysis during the observation period. The overall 5-year renal survival rate was 92.0%. On the other hand, 166 patients (22.6%) were in clinical remission. A group with mild proteinuria included 197 patients (26.8%). Forty-seven patients of this group showed minor glomerular abnormalities, whereas 12 patients with mild proteinuria showed severe mesangial involvement. Three patients with mild proteinuria were on dialysis during the observation period, whose proteinuria was increased during the clinical course. A group with minor glomerular abnormalities included 82 patients (11.2%). Forty-seven patients of this group showed mild proteinuria, of whom 12 patients showed moderate proteinuria. However, three patients with minor glomerular abnormalities who were not on dialysis showed loss of renal function. CONCLUSION: These results indicated the heterogeneity of the course and prognosis in IgAN. Even if a patient's initial clinical or histological findings are comparatively mild, strict follow-up management is needed.     

Carcinoid crisis and reversible right ventricular dysfunction after embolization in untreated carcinoid syndrome.

A 59-year-old white male with carcinoid tumor and hepatic metastases underwent hepatic artery embolization. The patient developed carcinoid crisis and a subsequent transthoracic echocardiogram showed classic findings of carcinoid heart disease along with a dilated hypertrophied right ventricle and severely depressed right ventricular ejection fraction. After treatment with octreotide the patient's clinical condition improved and a repeat transthoracic echocardiogram showed a significant improvement and normalization of right ventricular systolic function. Serotonin levels showed a progressive decline that correlated well with the patient's improved clinical condition. These findings suggest that the acute right ventricular dysfunction was secondary to acute carcinoid crisis and resolution resulted in a significant improvement of both right ventricular systolic function and clinical condition.     

Elevated platelet count features the variant type of BCR/ABL junction in chronic myelogenous leukaemia

A variant form of BCR/ABL junction was identified in a patient with chronic myelogenous leukaemia (CML). The BCR/ABL fusion mRNA of this patient showed in-frame junction between BCR exon c3 and ABL exon 2. Although the diagnosis of CML was made, the patient showed clinical features of essential thrombocythaemia (ET) rather than that of typical CML. Treatment with interferon-α showed no cytogenetic response. The c3-a2 type of BCR/ABL junction seems to be associated with elevated platelet count and thus could form a novel clinical entity different from typical CML.     

The clinical significance of classifications of cirrhosis. A comparison between conventional criteria and numerical taxonomy.

Classification of patients with cirrhosis serves the clinical purpose of selecting patients for adequate therapy. A material of 441 patients from a randomized clinical trial of prednisone treatment were classified according to different criteria, and the effect of this therapy was assessed by comparing the survival curves of controls and treated patients in the groups. Six classifications based on single criteria were studied, viz. females vs. males, alcoholics vs. non-alcoholics, compensated vs. decompensated cases, active vs. inactive cases, manifest vs. latent cases, and non-primary biliary vs. primary biliary cirrhosis. The latter group was too small for further analysis. Furthermore, the material was classified by numerical taxonomy, based on 56 clinical symptoms and signs and histological and serological changes. The taxonomical classification showed interdependence with all the clinical classifications, except that of primary biliary cirrhosis. Three clinical classifications, viz. those based on sex, alcoholism, and compensation, and one taxonomic class showed a significant therapeutic relevance. Combination of these criteria revealed that female patients with compensated non-alcoholic cirrhosis, previously demonstrated to benefit from prednisone treatment, showed a markedly greater prednisone effect if they belonged to the taxonomic group than if they did not. According to clinical, histologic, and laboratory data, the latter group was characterized as less severe cases than the former one. Whereas this discrepancy between clinical severity and therapeutic effect cannot be fully explained, the data do suggest that clinical criteria may not be ideal for selection of patients with cirrhosis for steroid therapy, and that numerical taxonomy may provide clues for more clinically significant classifications.     

Diagnosing patients with longstanding shoulder joint pain

OBJECTIVE: To examine the interobserver agreement of commonly used clinical tests and diagnoses in patients with shoulder pain, and the accuracy of these tests and ultrasonographic findings in comparison with arthroscopic findings. METHODS: Eighty six patients with longstanding shoulder joint pain were "blindly" examined by two trained doctors using several clinical tests. In all patients an ultrasonographic examination was performed, and in 42 (49%) an arthroscopy. RESULTS: Tests for impingement showed poor to moderate agreement. Tenderness of muscles, muscle weakness, and tests for labral lesion also showed poor agreement. Pain during muscle contraction showed moderate agreement. The agreement of clinical diagnoses was poor and the accuracy was low in comparison with arthroscopy. Ultrasonography was accurate in full thickness supraspinatus tendon tears, but inaccurate for partial tears and labral lesions. CONCLUSIONS: Most clinical tests showed poor agreement. Clinical and ultrasonographic diagnoses had low accuracy in comparison with arthroscopy.     

CT brain scanning in the diagnosis and localisation of cerebral schistosomiasis

CT scanning was performed on 25 cases of cerebral schistosomiasis. The images of the granuloma caused by schistosome eggs showed a high density mass shadow while the edema of the white matter showed a low density shadow. Combining CT scanning, with case history, clinical signs and immunologic assay we could make a correct diagnosis for each of the patients about the pathologic site and nature. The correlations between the images of CT scanning and clinical pathology were analysed and a better treatment regimen was discussed based on our experience. Some of the patients treated with praziquantel and checked by CT scanning showed pathologic changes of the granuloma disappeared during a follow-up period of 3-12 months.     

Ceftriaxone versus Levofloxacin for antibiotic therapy in patients with acute cholangitis

INTRODUCTION: For the therapy of acute cholangitis complete biliary drainage and antibiotic therapy is needed. The aim of the current study was to compare intravenous therapy of acute cholangitis with Ceftriaxone or Levofloxacin in a prospective and randomized fashion. METHODS: Patients with biliary obstruction and clinical signs of infection received in addition to 1.5 g Metronidazole either 500 mg Levofloxacin/die or 2 g Ceftriaxone/die. Early on during ERCP, bile was aspirated via the cannulation catheter and cultured for bacteria under aerobic and anaerobic conditions. Minimal inhibitory concentrations of the respective antibiotics were determinate for each isolate. The clinical course was followed for at least 6 days with clinical and laboratory data. RESULTS: 60 patients with clinical signs of acute cholangitis were randomised. In 40 patients (66 %) biliary colonization with bacteria could be identified. In all bacterial species Levofloxacin showed significantly lower rates of in-vitro resistance as compared to Ceftriaxone. However, the percentage of patients with a clinical cure or significant improvement was the same in the two groups. CONCLUSIONS: The clinical effect of Levofloxacin and Ceftriaxone in patients with acute cholangitis showed no significant differences. Because of improved in-vitro efficiency, a calculated therapy with Levofloxacin might be advantageous.     

心尖肥厚型心肌病的临床诊断探讨

目的 了解心尖肥厚型心肌病的临床表现和辅助检查特点。方法 总结２９例心尖肥厚型心肌病的临床表现和心电图,超声心动图,核素心肌断层显像,运动平板心电图及冠状动脉和左室造影的特征,确定心尖肥厚型心肌病的诊断方法。结果 心电图显示以胸导为主的导联Ｒ波振幅呈Ｖ４≥Ｖ５〉Ｖ３关系增高,同时伴有Ｔ波对称性深倒置,超声心动图和核素心肌断层显像显示心尖部肌肉肥厚,２０例活动平板心电图有心肌缺血,左心室造影心尖部肌     

Treatment of left-sided ulcerative colitis with 4-aminosalicylic acid enemas. A double-blind, placebo-controlled trial

Twenty-five patients with active left-sided ulcerative colitis were randomly assigned to receive either 2 g of 4-aminosalicylic acid (para-aminosalicylic acid) or placebo in a 60-mL volume as a nightly retention enema. The duration of treatment was 8 weeks. Disease activity was assessed by grading clinical symptoms of blood, mucus, urgency, sigmoidoscopic findings, and degree of histologic inflammation in rectal biopsies. At 8 weeks, 10 of 12 patients (83%; 95% confidence interval [CI], 55% to 97%) who received 4-aminosalicylic acid showed improvement in clinical, sigmoidoscopic, and histologic variables. In contrast, only 2 of 13 patients (15%, 95% CI, 4% to 38%) who had received placebo showed clinical improvement (P less than 0.005). The 11 patients in the placebo group who showed no improvement were treated subsequently with open-label 4-aminosalicylic acid enemas. Of the 11, 9 showed clinical, sigmoidoscopic, and histologic improvement. No adverse effects were seen. 4-Aminosalicylic acid enemas are a safe and effective means of treating left-sided ulcerative colitis.     

Hypersensitivity pneumonitis induced by toluene diisocyanate: sequelae of continuous exposure

A 41-year-old automobile paint sprayer showed the clinical features of hypersensitivity pneumonitis 1 week after he had begun to work with paint materials containing toluene diisocyanate. His symptoms began 6 to 8 hours after exposure to the agent and spontaneously disappeared by the next morning. He had diffuse, fine reticulonodular shadows on a chest roentgenogram and a restrictive impairment of pulmonary function. Immunoglobulin G antibody to toluene diisocyanate-human serum albumin was present in bronchoalveolar lavage fluid and sera: IgA antibody was present only in bronchoalveolar lavage fluid. Also, the patient had sensitized bronchoalveolar lymphocytes to toluene diisocyanate-human serum albumin. The histologic findings suggested hypersensitivity pneumonitis. The results of bronchoalveolar lavage, which was repeated on four separate occasions, showed lymphocytosis and a predominance of suppressor-cytotoxic T cells. The findings from serial determinations of humoral antibodies showed no changes consistent with the results of clinical and laboratory studies. In contrast, blastogenic responses of bronchoalveolar lymphocytes to toluene diisocyanate markedly decreased, and the patient showed clinical improvement despite continued exposure to the agent.     

ⅢA-N2期肺癌新辅助化疗前后Ki-67表达水平与预后关系

目的探讨ⅢA-N2期非小细胞肺癌(NSCLC)新辅助化疗(NCT)前后Ki-67表达水平与预后的关系。方法收集60例ⅢA-N2期NSCLC患者,首先进行新辅助化疗(NCT),再行手术。观察NCT疗效,比较Ki-67表达与临床疗效和预后的关系。结果NCT客观缓解率(ORR)是53.3%。NCT前后癌组织中Ki-67表达阳性率分别为63.2%和43.9%(P<0.05)。临床疗效显著组与疗效不显效组比较,Ki-67表达阳性率较低(31.3%vs 60.0%)(P<0.05)。生存分析显示:Ki-67表达阴性者具有更好的OS和PFS(P<0.01)。单因素分析示:Ki-67表达阴性者有更高的2年PFS和OS。多因素分析示:Ki-67阴性表达是预后的独立危险因素。结论Ki-67表达水平影响临床疗效和预后,Ki-67可作为NSCLC新辅助化疗疗效预测指标。     

Long-term follow-up after Carpentier tricuspid valvuloplasty

To study the long-term results of tricuspid valvuloplasty, pre- and postoperative (51 +/- 17 months) echocardiographic and catheterization data were collected from 51 patients (aged 59 +/- 9 years). Because of severe mitral stenosis all patients received a heterograft in the mitral position and underwent reconstructive tricuspid surgery with Carpentier rings. With regard to postoperative clinical outcome three patient groups were distinguished: 37 patients (group A) showed clear clinical improvement; in seven patients mild, and in three patients moderate tricuspid regurgitation persisted. A mild tricuspid stenosis of less than 4 mm Hg mean diastolic gradient was found in 11 patients. Patients in group B (n = 8) showed no clinical improvement, but there was persistence of moderate tricuspid regurgitation associated with nearly unchanged pulmonary hypertension in five patients and moderate tricuspid stenosis in two. Six patients (group C) showed deterioration of their clinical status; in two patients a severe degree of tricuspid regurgitation persisted, and four patients were first seen with a tricuspid stenosis with a mean diastolic gradient greater than 7 mm Hg. Analysis of postoperative data showed that tricuspid stenosis may develop during surgery in patients with slightly shrunken valve leaflets. Although the area of the anterior tricuspid leaflet was slightly underestimated, we found that long-term results of tricuspid valve annuloplasty with a Carpentier ring were encouraging. Doppler echocardiography for detection of tricuspid regurgitation and transvalvular pressure gradient showed results identical to hemodynamic data and is a suitable and sensitive method for evaluating postoperative results after tricuspid valve annuloplasty.     

Dramatic improvement of anti-SS-A/Ro-associated interstitial lung disease after immunosuppressive treatment

The aim of the study was to report three patients affected by interstitial lung disease associated with positive anti-SS-A/Ro autoantibody who showed a dramatic improvement after immunosuppressive treatment. Medical charts were reviewed to obtain clinical data, laboratory parameters, lung function tests, high-resolution computed tomography results and response to immunosuppressive treatment. The three patients showed a clinical picture of a lung-dominant connective tissue disease characterized by a sudden onset with dyspnea, cough and subtle extrathoracic features together with positive anti-SS-A/Ro antibody and weak titer antinuclear antibodies. All three patients responded favorably to immunosuppressive therapy: Two cases were treated with a combination of corticosteroid and cyclophosphamide followed by mycophenolate mofetil; in the third patient, clinical benefit was obtained after rituximab was added to corticosteroid and immunosuppressant drug. In spite of an abrupt onset with significant lung function impairment, all three patients had a favorable clinical response to immunosuppressive therapy. This report may be useful in making therapeutic decisions in case of interstitial lung disease associated with anti-SS-A antibody.     

An ovarian lipid cell tumour causing virilization and Cushing's syndrome

We report a 73-year-old woman with rapidly developing symptoms and signs of Cushing's syndrome and high urinary free cortisol. She was virilized and hirsute with testosterone levels which became exceptionally high. ACTH was suppressed and CT scan of the adrenals and pelvis showed no abnormality. Ultrasound scanning showed enlargement of the left ovary. Venous catheter studies suggested a left ovarian source. A 2-cm diameter lipid cell tumour was removed from the left ovary laparoscopically. Histology was initially benign, and clinical and biochemical cure of Cushing's syndrome was rapid. However, the neoplastic nature of the ovarian tumour was demonstrated 12 months later when peritoneal metastases were detected and there was then clinical and biochemical relapse.     

Die Trypsinhemmung in unausgewählten Sera eines poliklinischen Patientengutes

Serum-antitrypsin concentration was determined in 1901 non-selected patients of a Medical Polyclinic. History and clinical signs of chronic bronchitis were found in 126 of them (6.6%). Only 10 patients showed intermediate antitrypsin concentrations, 5 of them showed clinical and radiological evidence of emphysema. No severe hypo-antitrypsinemia such as is characteristic of patients with homozygous alpha₁-antitrypsin deficiency, was discovered. Among the remaining patients with chronic bronchitis, 74 had a normal and 47 an elevated antitrypsin concentration in serum.     

Osteoarticular scintigraphy in comparison with clinical evidence

Bone scintigraphy is a technique which is often resorted to in diagnostic rheumatology. There are few data on the effective relevance of bone scintigraphy in the evaluation of chronic inflammatory diseases of the joints. The aim of this study was to compare the results of bone scintigraphy with clinical evidence in patients with rheumatoid arthritis or osteoarthritis. Seventy-five patients were submitted to total body bone scintigraphy (44 rheumatoid arthritis, 31 osteoarthritis). The nuclear medicine specialist indicated the list of joints showing uptake. For the same patients a rheumatologist indicated the number of affected joints. The laboratory and clinical data were recorded. The patients were first stratified according to the prevalence of the clinical evidence and scintigraphic uptake. The distribution was found to be not significant. Only 5.3% of patients showed no uptake. Thirty-three patients had no clinical evidence of disease; among these, 30 showed joint uptake. Considering only the patients with clinical evidence, 97.6% showed joint uptake. These results were confirmed even when the data were analyzed by sex, disease and therapy. Considering the patients with clinical evidence, the uptake/clinical ratio did not show any significant correlation. The number of joints with clinical evidence correlated with the erythrocyte sedimentation rate. The number of joints showing uptake correlated only with age. In conclusion, on average, scintigraphy, performed in patients with rheumatoid arthritis and osteoarthritis, highlights a significantly higher number of joints involved as compared to what would be expected on the basis of clinical evaluation. It remains to be defined whether this is an overestimation related to the characteristics of the scan or whether it is sign of a higher sensitivity in highlighting the site of inflammation. Against the latter hypothesis is the absence of correlation with the inflammatory indexes.